Abstract：
:Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disease caused by a CAG-repeat expanding mutation in ATXN3. We generated induced pluripotent stem cells (iPSCs) from a SCA3 patient by electroporation of dermal fibroblasts with episomal plasmids encoding L-MYC, LIN28, SOX2, KLF4, OCT4 and short hairpin RNA targeting P53. The resulting iPSCs had normal karyotype, were free of genomically integrated episomal plasmids, expressed pluripotency markers, could differentiate into the three germ layers in vitro and retained the disease-causing ATXN3 mutation. This iPSC line could be useful for the investigation of SCA3 disease mechanisms.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Hansen SK,Borland H,Hasholt LF,Tümer Z,Nielsen JE,Rasmussen MA,Nielsen TT,Stummann TC,Fog K,Hyttel Pdoi
10.1016/j.scr.2016.02.040subject
Has Abstractpub_date
2016-05-01 00:00:00pages
553-6issue
3eissn
1873-5061issn
1876-7753pii
S1873-5061(16)00083-0journal_volume
16pub_type
杂志文章abstract：:Stem cells can differentiate into various body tissues and organs and thus are considered as promising tools for cell therapy and tissue engineering. Early passage stem cells have high differentiation ability compared to late passage stem cells. Thus, it is important to use early passage stem cells in cell therapy. He...
journal_title：Stem cell research
pub_type： 杂志文章
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更新日期：2019-07-01 00:00:00
abstract：:Human lymphoblast cells from a female and male patient diagnosed with Alzheimer's disease (AD) with different genotypes of a functional copy number variation (CNV) in the AD risk gene CR1 were used to generate integration-free induced pluripotent stem cells (iPSCs) employing episomal plasmids expressing OCT4, SOX2, NA...
journal_title：Stem cell research
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abstract：:Myeloid ecotropic viral integration site 1 (MEIS1) plays an essential role in the development of several embryonic organs, such as the central nervous system and eyes. To further investigate the role of MEIS1 in embryonic development, herein, we generated a MEIS1 homozygous knockout human embryonic stem cell (hESC) li...
journal_title：Stem cell research
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doi：10.1016/j.scr.2020.102069
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abstract：:Salisphere-derived adult epithelial cells have been used to improve saliva production of irradiated mouse salivary glands. Importantly, optimization of the cellular composition of salispheres could improve their regenerative capabilities. The Rho Kinase (ROCK) inhibitor, Y27632, has been used to increase the prolifera...
journal_title：Stem cell research
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doi：10.1016/j.scr.2019.101608
更新日期：2019-12-01 00:00:00
abstract：:It has been very difficult, if not impossible, to establish mouse induced pluripotent stem cells (iPSCs) from differentiated cells, such as fibroblasts, without leukemia inhibitory factor (LIF). We have established and maintained LIF-independent iPSCs for longer than 120 days with modified Oct4 along with Sox2, Klf4, ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.08.005
更新日期：2015-09-01 00:00:00
abstract：:Asparagine synthetase (ASNS) deficiency (ASNSD; MIM #615574) is a rare neurodevelopmental disorder caused by mutations in the ASNS gene. The ASNS gene maps to cytogenetic band 7q21.3 and is 35 kb long. ASNSD is characterised by congenital microcephaly, severely delayed psychomotor development, seizures, and hyperekple...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101583
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abstract：:GABAergic interneuron dysfunction has been implicated in temporal lobe epilepsy (TLE), autism, and schizophrenia. Inhibitory interneuron progenitors transplanted into the hippocampus of rodents with TLE provide varying degrees of seizure suppression. We investigated whether human embryonic stem cell (hESC)-derived int...
journal_title：Stem cell research
pub_type： 杂志文章
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更新日期：2018-12-01 00:00:00
abstract：:Crumbs homologue 1 (CRB1) mutations have been found in retinitis pigmentosa (RP) patients lead to severe retinal dystrophies. The human induced pluripotent stem (iPS) cell line CSUASOi003-A derived from peripheral blood mononuclear cells (PBMCs) of a patient carrying two heterozygous mutations (2249G>A p.G750D and c.2...
journal_title：Stem cell research
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doi：10.1016/j.scr.2020.101742
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abstract：:The Genea016 human embryonic stem cell line was derived from a donated, fully commercially consented ART blastocyst, through ICM outgrowth on inactivated human feeders. The line showed pluripotent cell morphology and genomic analysis verified a 46, XX karyotype and female Allele pattern through traditional karyotyping...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.11.009
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journal_title：Stem cell research
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abstract：:Fanconi anemia is a genetic bone marrow failure syndrome. The current treatment options are suboptimal and do not prevent the eventual onset of aplastic anemia requiring bone marrow transplantation. We previously showed that resveratrol, an antioxidant and an activator of the protein deacetylase Sirt1, enhanced hemato...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.05.007
更新日期：2015-07-01 00:00:00
abstract：:The peptide hormone insulin produced by pancreatic β-cells undergoes post-transcriptional processing before secretion. In particular, C-peptide is cleaved from pro-insulin to generate mature insulin. Here, we introduce a C-peptide-mCherry human iPSC line (HMGUi001-A-8). The line was generated by CRISPR/Cas9 mediated h...
journal_title：Stem cell research
pub_type： 杂志文章
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journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102002
更新日期：2020-10-01 00:00:00
abstract：:We report here the generation of human iPS cell line UKKi009-A from dermal fibroblasts of a patient carrying heterozygous mutation c.3035-3045delTCCCTCGATGC, p.Leu1012Pro (fs*55) in KCNH2 gene leading to long QT syndrome type 2 (LQT2). We used the Sleeping Beauty transposon-based plasmids expressing OSKM along with mi...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.12.039
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abstract：:Expanded human T cells from a Japanese female with recessive dystrophic epidermolysis bullosa (RDBE) were used to generate integration-free induced pluripotent stem cells (iPSCs) by exogenous expression of four reprogramming factors, OCT3/4, SOX2, cMYC, KLF4, using Sendai virus vector (SeVdp). The authenticity of esta...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.05.003
更新日期：2016-07-01 00:00:00
abstract：:In order to evaluate the functional significance of palmitoylation during multi-potent neural stem/progenitor cell proliferation and differentiation, retinoic acid-induced P19 cells were used in this study as a model system. Cell behaviour was monitored in the presence of the protein palmitoylation inhibitor 2-bromopa...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2013.12.010
更新日期：2014-03-01 00:00:00
abstract：:The outcomes of clinical trials using bone marrow stromal cell (BMSC) are variable; the degree of the expansion of BMSCs during clinical manufacturing may contribute to this variability since cell expansion is limited by senescence. Human BMSCs from aspirates of healthy subjects were subcultured serially until cell gr...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2013.07.005
更新日期：2013-11-01 00:00:00
abstract：:An important practical limitation of the three-dimensional geometry of stem-cell derived intestinal organoids is that it prevents easy access to the apical epithelium for testing food components, microorganisms, bioactive and toxic compounds. To this end, we here report on a new robust method for generating confluent ...
journal_title：Stem cell research
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doi：10.1016/j.scr.2018.02.013
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pub_type： 杂志文章
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journal_title：Stem cell research
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更新日期：2009-05-01 00:00:00
abstract：:Liver progenitor (oval) cells have enormous potential in the treatment of patients with liver disease using a cell therapy approach, but their use is limited by their scarcity and the number of donor livers from which they can be derived. Bone marrow may be a suitable source. Previously the derivation of oval cells fr...
journal_title：Stem cell research
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更新日期：2008-09-01 00:00:00
abstract：:Leucine rich repeat kinase (LRRK2) is the most prevalent genetic cause for Parkinson's disease. LRRK2 p.G2385R is an Asian specific genetic risk factor for sporadic Parkinson's disease. We generated two induced pluripotent stem cells (iPSCs), IBMS-iPSC-018-09 and IBMS-iPSC-020-01, from the peripheral blood mononuclear...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.01.034
更新日期：2018-04-01 00:00:00
abstract：:We report the engendering an isogenic iPSC line from the IBMS-iPSC-014-05 with homozygous correction of the R803X, Chr4: 88989098C > T in PKD2, using CRISPR/Cas9 technology. The results from the isogenic control, IBMS-iPSC-014-05C, showed that mutation had been corrected, while maintaining normal morphology, pluripote...
journal_title：Stem cell research
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abstract：:Neural stem cells (NSCs) have been considered as potential therapy in Alzheimer's disease (AD) but their use is hampered by the poor survival of grafted cells. Supply of neurotrophic factors to the grafted cells has been proposed as a way to augment survival of the stem cells. In this context, we investigated the util...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.04.008
更新日期：2015-07-01 00:00:00
abstract：:Two iPSC clones, NCCSi008-A and NCCSi008-B, were generated from a healthy male individual of Indian origin by reprogramming his CD4+ T cells with an integration free Sendai viral vector. The established iPSC clones showed high alkaline phosphatase (ALP) activity, expression of pluripotency markers, a normal male karyo...
journal_title：Stem cell research
pub_type： 杂志文章
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更新日期：2020-05-01 00:00:00
abstract：:Best's disease (BD) is an inherited retinal degenerative disease caused by mutations in BEST1 gene. A human induced pluripotent stem cell (iPSC) line has been generated with integration-free Sendai virus method from peripheral blood mononuclear cells (PBMCs) of a BD patient carrying c.888C > A mutation in BEST1 gene. ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101459
更新日期：2019-07-01 00:00:00
abstract：:Frontotemporal dementia with parkinsonism linked to chromosome 17q21.2 (FTDP-17) is an autosomal-dominant neurodegenerative disorder. Mutations in the MAPT (microtubule-associated protein tau) gene can cause FTDP-17, but the underlying pathomechanisms of the disease are still unknown. Induced pluripotent stem cells (i...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.09.024
更新日期：2016-11-01 00:00:00
abstract：:Mesenchymal stem cells (MSC) have been isolated from almost every adult tissue. In cord blood (CB), different non-hematopoietic CD45-, CD34- adherent cell populations can be generated: the cord blood derived MSC (CB-MSC), that behave almost like MSC from bone marrow (BM-MSC), and unrestricted somatic stem cells (USSC)...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2010.03.004
更新日期：2010-07-01 00:00:00
abstract：:Human iPSC line N44SV.5 was generated from primary normal human dermal fibroblasts belonging to the European mitochondrial haplogroup U. For this purpose, reprogramming factors Oct3/4, Sox2, Klf4, and cMyc were delivered using a non-integrative methodology that involves the use of Sendai virus. ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.12.010
更新日期：2016-01-01 00:00:00
